利妥昔单抗在儿童原发性肾病综合征的应用新进展

New Progress in the Application of Rituximab in Children with Primary Nephrotic Syndrome

原发性肾病综合征(Primary nephrotic syndrome, PNS)是儿童最常见的肾小球疾病,其发病与自身免疫有关,约25%的患者表现为频复发或激素依赖、激素耐药,进而发展成难治性肾病综合征,以单独的糖皮质激素或联合免疫抑制剂治疗为主。利妥昔单抗(rituximab,RTX)是一种诱导B细胞溶解和凋亡的嵌合抗CD20单克隆抗体,能够预防或延缓儿童原发性肾病综合征患者的复发,延缓进入终末期肾病。近年来,已有多个病例回顾性研究和随机对照试验证实利妥昔单抗在治疗原发性肾病综合的有效性和安全性,极大的改善了儿童原发性肾病综合征的预后。本文就RTX的作用机制、在儿童PNS临床应用疗效及不良反应进行综述。

Primary nephrotic syndrome (PNS) is the most common glomerular disease in children. Its onset is related to autoimmunity. Approximately 25% of patients show frequent relapses or hormone dependence and hormone resistance, and then develop into refractory nephrotic syndrome which is mainly treated with glucocorticoid alone or combined with immunosuppressive agents. Rituximab (RTX) is a chimeric anti-CD20 monoclonal antibody that induces B cell lysis and apoptosis. It can prevent or delay the recurrence of children with primary nephrotic syndrome and delay the entry into end-stage renal disease. In recent years, retrospective studies and randomized controlled trials of multiple cases have confirmed the effectiveness and safety of rituximab in the treatment of primary nephrotic syndrome, which has greatly improved the prognosis of children with primary nephrotic syndrome. This article reviews the mechanism of action of RTX, its clinical efficacy and adverse reactions in Children PNS.